Three subjects (ED-01 IV-4 and IV-5 and ED-02 VI-3) had deafness that developed in early infancy (c.f. the later-onset deafness in other families with GRHL2 haploinsufficiency).15,16 Of note, none of the heterozygous carriers of either missense mutation in GRHL2 had any deafness. The gene discussed is GRHL2; the disease is deafness.